Premium
Congenital Tuftsin Deficiency
Author(s) -
CONSTANTOPOULOS ANDREAS
Publication year - 1983
Publication title -
annals of the new york academy of sciences
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.1983.tb37106.x
Subject(s) - tuftsin , tetrapeptide , medicine , immunology , gastroenterology , peptide , chemistry , biochemistry
The serum tuftsin activity in four different pediatric groups has been studied: (a) 20 premature infants, (b) 20 full-term infants, (c) 20 normal children (controls), and (d) 5 patients with recurrent and severe infections of the respiratory system, skin, and lymph nodes. The normal children as well as the premature and full-term infants had normal tuftsin activity. Tuftsin activity in the serum of the 5 patients in group (d) was absent. Cellular and humoral immunity in these patients was normal. All patients have been shown to have a mutant peptide that is strongly inhibitory to the normal tetrapeptide tuftsin. The clinical response of these patients to gamma-globulin was excellent.